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  • Title: Radiation therapy for choroid plexus carcinoma patients with Li-Fraumeni syndrome: advantageous or detrimental?
    Author: Bahar M, Kordes U, Tekautz T, Wolff J.
    Journal: Anticancer Res; 2015 May; 35(5):3013-7. PubMed ID: 25964589.
    Abstract:
    BACKGROUND: Choroid plexus carcinomas (CPCs) are rare pediatric tumors often associated with Li-Fraumeni Syndrome (LFS), a germline mutation in the TP53 tumor-suppressor gene, predisposing to cancer. MATERIALS AND METHODS: We performed a systemic literature review from 1990-2013 to evaluate the hypothesis that radiation therapy should be avoided in patients with CPC and LFS. Overall survival (OS) was compared using Kaplan-Meier curves and log-rank tests. RESULTS: Twenty-eight patients were documented with CPC and LFS. Eleven out of 17 patients received radiation therapy. The survival of patients receiving radiation was inferior to that of those without radiation [median (±95% confidence interval) 2-year OS=0.18 ± 0.12% versus 0.58 ± 0.12%]. The log-rank tests suggested the difference to be marginally significant (p=0.056). CONCLUSION: This finding provides evidence for pursuing treatment approaches that do not include radiation therapy for patients with LFS.
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